You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-04-16 17:02:02 UTC
HMDB IDHMDB0000254
Secondary Accession Numbers
  • HMDB00254
Metabolite Identification
Common NameSuccinic acid
DescriptionSuccinic acid is a dicarboxylic acid. The anion, succinate, is a component of the citric acid or TCA cycle and is capable of donating electrons to the electron transfer chain. Succinate dehydrogenase (SDH) plays an important role in mitochondrial function, being both part of the respiratory chain and the Krebs cycle. SDH with a covalently attached FAD prosthetic group, is able to bind several different enzyme substrates (succinate and fumarate) and physiological regulators (oxaloacetate and ATP). Oxidizing succinate links SDH to the fast-cycling Krebs cycle portion where it participates in the breakdown of acetyl-CoA throughout the entire Krebs cycle. Succinate can readily be imported into the mitochondrial matrix by the n-butylmalonate- (or phenylsuccinate-) sensitive dicarboxylate carrier in exchange with inorganic phosphate or another organic acid, e. g. malate (PMID 16143825 ). Mutations in the four genes encoding the subunits of the mitochondrial succinate dehydrogenase are associated with a wide spectrum of clinical presentations, i.e.: Huntington's disease (PMID 11803021 ). Moreover, succinic acid is found to be associated with D-2-hydroxyglutaric aciduria, which is an inborn error of metabolism. Succinic acid has recently been identified as an oncometabolite or an endogenous, cancer causing metabolite. High levels of this organic acid can be found in tumors or biofluids surrounding tumors. Its oncogenic action appears to due to its ability to inhibit prolyl hydroxylase-containing enzymes. In many tumours, oxygen availability becomes limited (hypoxia) very quickly due to rapid cell proliferation and limited blood vessel growth. The major regulator of the response to hypoxia is the HIF transcription factor (HIF-alpha). Under normal oxygen levels, protein levels of HIF-alpha are very low due to constant degradation, mediated by a series of post-translational modification events catalyzed by the prolyl hydroxylase domain-containing enzymes PHD1, 2 and 3, (also known as EglN2, 1 and 3) that hydroxylate HIF-alpha and lead to its degradation. All three of the PHD enzymes are inhibited by succinate. Succinic acid is found to be associated with D-2-hydroxyglutaric aciduria, which is an inborn error of metabolism.
Structure
Thumb
Synonyms
ValueSource
1,2-Ethanedicarboxylic acidChEBI
Acide butanedioiqueChEBI
Acide succiniqueChEBI
Acidum succinicumChEBI
Amber acidChEBI
AsuccinChEBI
BernsteinsaeureChEBI
ButandisaeureChEBI
Butanedionic acidChEBI
Dihydrofumaric acidChEBI
e363ChEBI
Ethylenesuccinic acidChEBI
HOOC-CH2-CH2-COOHChEBI
Spirit OF amberChEBI
1,2-EthanedicarboxylateGenerator
SuccinateGenerator
ButanedionateGenerator
DihydrofumarateGenerator
EthylenesuccinateGenerator
1,4-ButanedioateHMDB
1,4-Butanedioic acidHMDB
KatasuccinHMDB
Wormwood acidHMDB
1,2 Ethanedicarboxylic acidMeSH
1,4 Butanedioic acidMeSH
Potassium succinateMeSH
Succinate, ammoniumMeSH
Butanedioic acidMeSH
Succinate, potassiumMeSH
Ammonium succinateMeSH
Chemical FormulaC4H6O4
Average Molecular Weight118.088
Monoisotopic Molecular Weight118.02660868
IUPAC Namebutanedioic acid
Traditional Namesuccinic acid
CAS Registry Number110-15-6
SMILES
OC(=O)CCC(O)=O
InChI Identifier
InChI=1S/C4H6O4/c5-3(6)1-2-4(7)8/h1-2H2,(H,5,6)(H,7,8)
InChI KeyKDYFGRWQOYBRFD-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassDicarboxylic acids and derivatives
Direct ParentDicarboxylic acids and derivatives
Alternative Parents
Substituents
  • Fatty acid
  • Dicarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Biological role:

Industrial application:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point185 - 188 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility83.2 mg/mLNot Available
LogP-0.59HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility211 g/LALOGPS
logP-0.53ALOGPS
logP-0.4ChemAxon
logS0.25ALOGPS
pKa (Strongest Acidic)3.55ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area74.6 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity23.54 m³·mol⁻¹ChemAxon
Polarizability10.14 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0002-0920000000-f286e6204a4163b823baView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0002-0900000000-bf336910bb37d7f78140View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-006t-9800000000-df5ff4e8457d2d4ef919View in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-00c1-3930000000-3cc18e719822b5af661aView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-004i-9000000000-93b4807ae6275a3e59d7View in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0kos-9100000000-f1df0903a24c305e68ecView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0002-0910000000-300c33b39fb991b5a73eView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0920000000-f286e6204a4163b823baView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0900000000-bf336910bb37d7f78140View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-006t-9800000000-df5ff4e8457d2d4ef919View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00c1-3930000000-3cc18e719822b5af661aView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0900000000-f838d863ee7c2b111f02View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-00xr-9300000000-f0644daf4fbb11fcc2dcView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00dj-9710000000-ff8325384b9eefd19106View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated)splash10-00di-9300000000-f9dc864d93a09d3074f9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated)splash10-00di-9300000000-76c151de384928b2256fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated)splash10-01b9-7900000000-51d2341c097f04827944View in MoNA
LC-MS/MSLC-MS/MS Spectrum - EI-B (Unknown) , Positivesplash10-004i-9000000000-93b4807ae6275a3e59d7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-03dj-0971010000-37d214dc7a8fdc26116bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-014i-9000000000-249222ac742c1634cec9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-00di-9000000000-6897d49472dba6a34a27View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-0udi-0490000000-d138f8023125921b4b82View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-014i-1900000000-4ffdabe5bde527b66982View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-00di-9100000000-c20baa818f5ff5f678c1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-00di-9000000000-7a49a18aa6fcb2540a12View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-00di-9000000000-9955aeb0e5a9f88ae70eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-00di-9000000000-7e1f195f111b4eafb4faView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-00xr-9400000000-e50afc90e20cd420ba9bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-00xr-9600000000-43167f2549cbb5d5f7e8View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-014i-1900000000-4ffdabe5bde527b66982View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-9100000000-7a8bfa543dc087bea06dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-9000000000-fdec6c7458176f3cbeb8View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-9000000000-9955aeb0e5a9f88ae70eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0gb9-2900000000-9d959a53833b07094158View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0l6r-9600000000-c367e11e737714d41418View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-056r-9000000000-e65aa602a8293debec36View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-014i-2900000000-e1f840494c9003279869View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-014i-7900000000-1089efd4a3469bcf14f1View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0604-9000000000-0e0e60bb202ffb004894View in MoNA
MSMass Spectrum (Electron Ionization)splash10-05di-9100000000-c629bea41d0d3d896425View in MoNA
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,1H] 2D NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
  • Endoplasmic reticulum
  • Peroxisome
Biospecimen Locations
  • Blood
  • Breast Milk
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Location
  • Adipose Tissue
  • Brain
  • Fibroblasts
  • Kidney
  • Liver
  • Muscle
  • Pancreas
  • Placenta
  • Prostate
  • Skeletal Muscle
  • Spleen
Pathways
NameSMPDB/PathwhizKEGG
2-Hydroxyglutric Aciduria (D And L Form)ThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
2-ketoglutarate dehydrogenase complex deficiencyThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase DeficiencyThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase DeficiencyThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
3-hydroxyisobutyric acid dehydrogenase deficiencyThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified16.0 (0.00-32.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified23.5 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified8.8 +/- 2.7 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified6.10-11.5 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Breast MilkDetected and Quantified8.4 +/- 2.9 uMAdult (>18 years old)Female
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified47.2 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified19 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified150.0 +/- 110.0 uMElderly (>65 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified3 +/- 2 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified28.5 (24-33) uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified3.0 +/- 2.0 uMAdult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)MaleNormal details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)FemaleNormal details
FecesDetected but not Quantified Infant (0-1 year old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Not Specified
Normal
details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)FemaleNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected and Quantified132.528 +/- 151.836 nmol/g wet fecesNot SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected and Quantified1633.528 +/- 1822.370 nmol/g wet fecesNot SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedNormal details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
FecesDetected and Quantified255 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected and Quantified222 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal
details
FecesDetected and Quantified250 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal
details
SalivaDetected and Quantified125.15 +/- 180.60 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified2260 (60.0-4460) uMAdult (>18 years old)BothNormal details
SalivaDetected and Quantified14 (2-112) uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified9 (1-78) uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified21 (<1-66) uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified9 (<1-54) uMAdult (>18 years old)Female
Normal
details
SalivaDetected and Quantified9.03 +/- 5.22 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected and Quantified15.0 +/- 15.5 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified15.6 +/- 27.7 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected and Quantified24.3 +/- 20.4 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified4-145 uMAdult (>18 years old)Male
normal
details
SalivaDetected and Quantified1-33 uMAdult (>18 years old)Male
normal
details
SweatDetected but not Quantified Adult BothNormal details
UrineDetected but not Quantified Adult (>18 years old)MaleNormal details
UrineDetected and Quantified4.7 (1.1-14.5) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified12.60 +/- 12.13 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified14.48 +/- 3.20 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified<76 umol/mmol creatinineNot SpecifiedNot Specified
Normal
details
UrineDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected and Quantified7.7 (1.9-20.0) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified3.8 (1.25-6.7) umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected but not Quantified Adult (>18 years old)BothNormal details
UrineDetected and Quantified1.4-2.3 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified14.581 +/- 9.663 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified2.10 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Shaykhutdinov RA,...
details
UrineDetected and Quantified<87.14 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified9.9 +/- 5.0 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified14.4 +/- 4.9 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified7.5 (0.5-16) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified6.0 (0.3-33.3) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected but not Quantified Adult (>18 years old)Male
Normal
details
UrineDetected but not Quantified Adult (>18 years old)Male
Normal
details
UrineDetected and Quantified5.6 +/- 3.8 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified197.2 (29.4-486.2) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified185.4 (6.0-342.6) umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified11.6 (4.0-27.3) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified8.25 (0.5-16.0) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.4-1.7 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified163.491 +/- 191.759 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified<335.0976 umol/mmol creatinineInfant (0-1 year old)MaleNormal details
UrineDetected and Quantified1.915-71.807 umol/mmol creatinineChildren (1 - 13 years old)MaleNormal details
UrineDetected and Quantified1.3 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<97 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified6.2 (2.5-13.5) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified5.3 (5.6) uMAdult (>18 years old)FemaleDown syndrome pregnancy details
BloodDetected and Quantified5.6 (12.5) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified6.90 +/- 6.33 uMAdult (>18 years old)Female
Pregnancy with fetuses with trisomy 18
details
BloodDetected and Quantified6.50 +/- 10.83 uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified4.3 (1.6) uMAdult (>18 years old)FemaleEarly preeclampsia details
BloodDetected and Quantified12.4 (13.9) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified3.35 (1.78) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified3.31 (1.13) uMAdult (>18 years old)FemalePregnancy details
Cerebrospinal Fluid (CSF)Detected and Quantified19.0 (0.0-38.0) uMAdult (>18 years old)BothCanavan Disease details
Cerebrospinal Fluid (CSF)Detected and Quantified55.0 (19.0-91.0) uMAdult (>18 years old)BothAlzheimer's disease details
FecesDetected but not Quantified Adult (>18 years old)MaleGout details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
FecesDetected but not Quantified Infant (0-1 year old)Not SpecifiedGenetically At-Risk of celiac disease details
FecesDetected but not Quantified Not SpecifiedNot Specified
Cryptosporidium infection
details
FecesDetected but not Quantified Adult (>18 years old)FemaleMyalgic encephalomyelitis/chronic fatigue syndrome details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not Quantified Adult (>18 years old)Both
Irritable bowel syndrome
details
FecesDetected but not Quantified Adult (>18 years old)Both
Ulcerative colitis
details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedCrohns disease details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedUlcerative colitis details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedUnclassified IBD details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
FecesDetected but not Quantified Adult (>18 years old)BothCrohn's disease details
UrineDetected and Quantified749-784 umol/mmol creatinineInfant (0-1 year old)MaleD-2-hydroxyglutaric aciduria details
UrineDetected but not Quantified Adult (>18 years old)FemaleEpithelial ovarian cancer details
UrineDetected and Quantified75-185 umol/mmol creatinineInfant (0-1 year old)Male
2-Ketoglutarate dehydrogenase complex deficiency
details
UrineDetected and Quantified43.35 +/- 25.08 umol/mmol creatinineChildren (1-13 years old)BothMalnutrition (type kwashiorkor and marasmus) details
UrineDetected and Quantified27.897 +/- 29.998 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified9.0 +/- 6.0 umol/mmol creatinineAdult (>18 years old)BothLung cancer details
UrineDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
UrineDetected and Quantified4.787 umol/mmol creatinineChildren (1 - 13 years old)MaleD-2-Hydroxyglutaric aciduria details
UrineDetected and Quantified467.612 +/- 705.456 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified6.667 umol/mmol creatinineChildren (1 - 13 years old)MaleD-2-Hydroxyglutaric aciduria details
UrineDetected and Quantified530.857 +/- 876.714 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
details
UrineDetected and Quantified50 umol/mmol creatinineInfant (0-1 year old)MaleMalonyl-Coa decarboxylase deficiency details
UrineDetected and Quantified86.168 umol/mmol creatinineChildren (1 - 13 years old)MaleD-2-Hydroxyglutaric aciduria details
UrineDetected and Quantified141.698 umol/mmol creatinineInfant (0-1 year old)MaleFumarase deficiency details
UrineDetected and Quantified15 umol/mmol creatinineChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
Associated Disorders and Diseases
Disease References
2-Ketoglutarate dehydrogenase complex deficiency
  1. Bonnefont JP, Chretien D, Rustin P, Robinson B, Vassault A, Aupetit J, Charpentier C, Rabier D, Saudubray JM, Munnich A: Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis. J Pediatr. 1992 Aug;121(2):255-8. [PubMed:1640293 ]
Alzheimer's disease
  1. Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. [PubMed:9693263 ]
Canavan disease
  1. Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. [PubMed:7729054 ]
D-2-hydroxyglutaric aciduria
  1. Chalmers RA, Lawson AM, Watts RW, Tavill AS, Kamerling JP, Hey E, Ogilvie D: D-2-hydroxyglutaric aciduria: case report and biochemical studies. J Inherit Metab Dis. 1980;3(1):11-5. [PubMed:6774165 ]
  2. Wajne M, Vargas CR, Funayama C, Fernandez A, Elias ML, Goodman SI, Jakobs C, van der Knaap MS: D-2-Hydroxyglutaric aciduria in a patient with a severe clinical phenotype and unusual MRI findings. J Inherit Metab Dis. 2002 Feb;25(1):28-34. [PubMed:11999977 ]
Lung Cancer
  1. Wishart DS, Knox C, Guo AC, Eisner R, Young N, Gautam B, Hau DD, Psychogios N, Dong E, Bouatra S, Mandal R, Sinelnikov I, Xia J, Jia L, Cruz JA, Lim E, Sobsey CA, Shrivastava S, Huang P, Liu P, Fang L, Peng J, Fradette R, Cheng D, Tzur D, Clements M, Lewis A, De Souza A, Zuniga A, Dawe M, Xiong Y, Clive D, Greiner R, Nazyrova A, Shaykhutdinov R, Li L, Vogel HJ, Forsythe I: HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 Jan;37(Database issue):D603-10. doi: 10.1093/nar/gkn810. Epub 2008 Oct 25. [PubMed:18953024 ]
Lipoyltransferase 1 Deficiency
  1. Soreze Y, Boutron A, Habarou F, Barnerias C, Nonnenmacher L, Delpech H, Mamoune A, Chretien D, Hubert L, Bole-Feysot C, Nitschke P, Correia I, Sardet C, Boddaert N, Hamel Y, Delahodde A, Ottolenghi C, de Lonlay P: Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase. Orphanet J Rare Dis. 2013 Dec 17;8:192. doi: 10.1186/1750-1172-8-192. [PubMed:24341803 ]
Associated OMIM IDs
  • 271900 (Canavan disease)
  • 104300 (Alzheimer's disease)
  • 211980 (Lung Cancer)
  • 600721 (D-2-hydroxyglutaric aciduria)
  • 203740 (2-Ketoglutarate dehydrogenase complex deficiency)
  • 616299 (Lipoyltransferase 1 Deficiency)
DrugBank IDDB00139
Phenol Explorer Compound IDNot Available
FoodDB IDFDB001931
KNApSAcK IDC00001205
Chemspider ID1078
KEGG Compound IDC00042
BioCyc IDSUC
BiGG ID33633
Wikipedia LinkSuccinic_acid
METLIN ID114
PubChem Compound1110
PDB IDSIN
ChEBI ID15741
References
Synthesis ReferenceBerglund, Kris Arvid; Andersson, Christian; Rova, Ulrika. Process for the production of succinic acid. PCT Int. Appl. (2007), 30pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
  2. Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed:8087979 ]
  3. Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [PubMed:8412012 ]
  4. Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. [PubMed:12097436 ]
  5. Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. [PubMed:9693263 ]
  6. Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. [PubMed:7729054 ]
  7. Magera MJ, Helgeson JK, Matern D, Rinaldo P: Methylmalonic acid measured in plasma and urine by stable-isotope dilution and electrospray tandem mass spectrometry. Clin Chem. 2000 Nov;46(11):1804-10. [PubMed:11067816 ]
  8. Zhang TM, Sener A, Malaisse WJ: Hydrolysis of succinic acid dimethyl ester in rat pancreatic islets. Biochem Mol Med. 1995 Aug;55(2):131-7. [PubMed:7582870 ]
  9. Groenen PM, Engelke UF, Wevers RA, Hendriks JC, Eskes TK, Merkus HM, Steegers-Theunissen RP: High-resolution 1H NMR spectroscopy of amniotic fluids from spina bifida fetuses and controls. Eur J Obstet Gynecol Reprod Biol. 2004 Jan 15;112(1):16-23. [PubMed:14687733 ]
  10. Meijer-Severs GJ, van Santen E: Short-chain fatty acids and succinate in feces of healthy human volunteers and their correlation with anaerobe cultural counts. Scand J Gastroenterol. 1987 Aug;22(6):672-6. [PubMed:3659829 ]
  11. Ren LC, Huang XY, Long JH: [Effects of succinic acid on the function of in vitro cultured human fibroblasts]. Zhonghua Shao Shang Za Zhi. 2004 Feb;20(1):34-6. [PubMed:15059451 ]
  12. Wevers RA, Engelke U, Heerschap A: High-resolution 1H-NMR spectroscopy of blood plasma for metabolic studies. Clin Chem. 1994 Jul;40(7 Pt 1):1245-50. [PubMed:8013094 ]
  13. Borenstein DG, Gibbs CA, Jacobs RP: Gas-liquid chromatographic analysis of synovial fluid: volatile short-chain fatty acids in septic arthritis. Ann Rheum Dis. 1983 Aug;42(4):362-7. [PubMed:6882030 ]
  14. Frenkel G, Peterson RN, Freund M: Oxidative and glycolytic metabolism of semen components by washed guinea pig spermatozoa. Fertil Steril. 1975 Feb;26(2):144-7. [PubMed:1126459 ]
  15. Briere JJ, Favier J, El Ghouzzi V, Djouadi F, Benit P, Gimenez AP, Rustin P: Succinate dehydrogenase deficiency in human. Cell Mol Life Sci. 2005 Oct;62(19-20):2317-24. [PubMed:16143825 ]
  16. Rustin P, Rotig A: Inborn errors of complex II--unusual human mitochondrial diseases. Biochim Biophys Acta. 2002 Jan 17;1553(1-2):117-22. [PubMed:11803021 ]

Only showing the first 10 proteins. There are 44 proteins in total.

Enzymes

General function:
Involved in succinate dehydrogenase activity
Specific function:
Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
Gene Name:
SDHC
Uniprot ID:
Q99643
Molecular weight:
16650.185
Reactions
Ubiquinone-2 + Succinic acid → QH2 + Fumaric aciddetails
References
  1. Bayley JP, van Minderhout I, Weiss MM, Jansen JC, Oomen PH, Menko FH, Pasini B, Ferrando B, Wong N, Alpert LC, Williams R, Blair E, Devilee P, Taschner PE: Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma. BMC Med Genet. 2006 Jan 11;7:1. [PubMed:16405730 ]
  2. Leibowitz G, Khaldi MZ, Shauer A, Parnes M, Oprescu AI, Cerasi E, Jonas JC, Kaiser N: Mitochondrial regulation of insulin production in rat pancreatic islets. Diabetologia. 2005 Aug;48(8):1549-59. Epub 2005 Jun 29. [PubMed:15986240 ]
  3. Bayley JP, Devilee P, Taschner PE: The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency. BMC Med Genet. 2005 Nov 16;6:39. [PubMed:16288654 ]
  4. Szeto SS, Reinke SN, Sykes BD, Lemire BD: Ubiquinone-binding site mutations in the Saccharomyces cerevisiae succinate dehydrogenase generate superoxide and lead to the accumulation of succinate. J Biol Chem. 2007 Sep 14;282(37):27518-26. Epub 2007 Jul 18. [PubMed:17636259 ]
  5. Kubo Y, Takagi H, Nakamori S: Effect of gene disruption of succinate dehydrogenase on succinate production in a sake yeast strain. J Biosci Bioeng. 2000;90(6):619-24. [PubMed:16232921 ]
General function:
Involved in electron carrier activity
Specific function:
Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Can act as a tumor suppressor.
Gene Name:
SDHA
Uniprot ID:
P31040
Molecular weight:
72690.975
Reactions
Succinic acid + Coenzyme Q10 → Fumaric acid + QH(2)details
Ubiquinone-2 + Succinic acid → QH2 + Fumaric aciddetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
  3. Maklashina E, Iverson TM, Sher Y, Kotlyar V, Andrell J, Mirza O, Hudson JM, Armstrong FA, Rothery RA, Weiner JH, Cecchini G: Fumarate reductase and succinate oxidase activity of Escherichia coli complex II homologs are perturbed differently by mutation of the flavin binding domain. J Biol Chem. 2006 Apr 21;281(16):11357-65. Epub 2006 Feb 15. [PubMed:16484232 ]
  4. Takeo S, Kokaze A, Ng CS, Mizuchi D, Watanabe JI, Tanabe K, Kojima S, Kita K: Succinate dehydrogenase in Plasmodium falciparum mitochondria: molecular characterization of the SDHA and SDHB genes for the catalytic subunits, the flavoprotein (Fp) and iron-sulfur (Ip) subunits. Mol Biochem Parasitol. 2000 Apr 15;107(2):191-205. [PubMed:10779596 ]
  5. Ackrell BA: Cytopathies involving mitochondrial complex II. Mol Aspects Med. 2002 Oct;23(5):369-84. [PubMed:12231007 ]
General function:
Involved in iron ion binding
Specific function:
Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (By similarity).
Gene Name:
SDHD
Uniprot ID:
O14521
Molecular weight:
17042.82
Reactions
Ubiquinone-2 + Succinic acid → QH2 + Fumaric aciddetails
References
  1. Brink I, Schaefer O, Walz M, Neumann HP: Fluorine-18 DOPA PET imaging of paraganglioma syndrome. Clin Nucl Med. 2006 Jan;31(1):39-41. [PubMed:16374125 ]
  2. Sun F, Huo X, Zhai Y, Wang A, Xu J, Su D, Bartlam M, Rao Z: Crystal structure of mitochondrial respiratory membrane protein complex II. Cell. 2005 Jul 1;121(7):1043-57. [PubMed:15989954 ]
  3. Bayley JP, Devilee P, Taschner PE: The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency. BMC Med Genet. 2005 Nov 16;6:39. [PubMed:16288654 ]
  4. Lehtonen HJ, Makinen MJ, Kiuru M, Laiho P, Herva R, van Minderhout I, Hogendoorn PC, Cornelisse C, Devilee P, Launonen V, Aaltonen LA: Increased HIF1 alpha in SDH and FH deficient tumors does not cause microsatellite instability. Int J Cancer. 2007 Sep 15;121(6):1386-9. [PubMed:17520677 ]
  5. Bayley JP, van Minderhout I, Weiss MM, Jansen JC, Oomen PH, Menko FH, Pasini B, Ferrando B, Wong N, Alpert LC, Williams R, Blair E, Devilee P, Taschner PE: Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma. BMC Med Genet. 2006 Jan 11;7:1. [PubMed:16405730 ]
General function:
Involved in electron carrier activity
Specific function:
Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
Gene Name:
SDHB
Uniprot ID:
P21912
Molecular weight:
31629.365
Reactions
Succinic acid + Coenzyme Q10 → Fumaric acid + QH(2)details
Ubiquinone-2 + Succinic acid → QH2 + Fumaric aciddetails
References
  1. Arikawa Y, Kuroyanagi T, Shimosaka M, Muratsubaki H, Enomoto K, Kodaira R, Okazaki M: Effect of gene disruptions of the TCA cycle on production of succinic acid in Saccharomyces cerevisiae. J Biosci Bioeng. 1999;87(1):28-36. [PubMed:16232421 ]
  2. Bayley JP, Devilee P, Taschner PE: The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency. BMC Med Genet. 2005 Nov 16;6:39. [PubMed:16288654 ]
  3. Lehtonen HJ, Makinen MJ, Kiuru M, Laiho P, Herva R, van Minderhout I, Hogendoorn PC, Cornelisse C, Devilee P, Launonen V, Aaltonen LA: Increased HIF1 alpha in SDH and FH deficient tumors does not cause microsatellite instability. Int J Cancer. 2007 Sep 15;121(6):1386-9. [PubMed:17520677 ]
  4. Bayley JP, van Minderhout I, Weiss MM, Jansen JC, Oomen PH, Menko FH, Pasini B, Ferrando B, Wong N, Alpert LC, Williams R, Blair E, Devilee P, Taschner PE: Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma. BMC Med Genet. 2006 Jan 11;7:1. [PubMed:16405730 ]
  5. Szeto SS, Reinke SN, Sykes BD, Lemire BD: Ubiquinone-binding site mutations in the Saccharomyces cerevisiae succinate dehydrogenase generate superoxide and lead to the accumulation of succinate. J Biol Chem. 2007 Sep 14;282(37):27518-26. Epub 2007 Jul 18. [PubMed:17636259 ]
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA).
Gene Name:
ALDH5A1
Uniprot ID:
P51649
Molecular weight:
57214.23
Reactions
Succinic acid semialdehyde + NAD + Water → Succinic acid + NADHdetails
Succinic acid semialdehyde + NAD + Water → Succinic acid + NADH + Hydrogen Iondetails
References
  1. Yogeeswari P, Sriram D, Vaigundaragavendran J: The GABA shunt: an attractive and potential therapeutic target in the treatment of epileptic disorders. Curr Drug Metab. 2005 Apr;6(2):127-39. [PubMed:15853764 ]
  2. Popov VN, Eprintsev AT, Fedorin DN, Fomenko OIu, Igamberdiev AU: [Role of transamination in the mobilization of respiratory substrates in germinating seeds of castor oil plants]. Prikl Biokhim Mikrobiol. 2007 May-Jun;43(3):376-81. [PubMed:17619587 ]
  3. Wang C, Zhang HB, Wang LH, Zhang LH: Succinic semialdehyde couples stress response to quorum-sensing signal decay in Agrobacterium tumefaciens. Mol Microbiol. 2006 Oct;62(1):45-56. Epub 2006 Aug 30. [PubMed:16942602 ]
  4. Ahn SJ, Yang CH, Cooksey DA: Pseudomonas putida 06909 genes expressed during colonization on mycelial surfaces and phenotypic characterization of mutants. J Appl Microbiol. 2007 Jul;103(1):120-32. [PubMed:17584458 ]
  5. Chiribau CB, Mihasan M, Ganas P, Igloi GL, Artenie V, Brandsch R: Final steps in the catabolism of nicotine. FEBS J. 2006 Apr;273(7):1528-36. [PubMed:16689938 ]
  6. Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [PubMed:11752352 ]
General function:
Involved in electron carrier activity
Specific function:
Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
Gene Name:
PHYH
Uniprot ID:
O14832
Molecular weight:
38538.065
Reactions
Phytanoyl-CoA + Oxoglutaric acid + Oxygen → 2-Hydroxyphytanoyl-CoA + Succinic acid + CO(2)details
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Gene Name:
P4HA2
Uniprot ID:
O15460
Molecular weight:
60632.19
Reactions
L-proline-[procollagen] + Oxoglutaric acid + Oxygen → trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2)details
L-Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Succinic acid + Carbon dioxidedetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Gene Name:
P4HA1
Uniprot ID:
P13674
Molecular weight:
60966.645
Reactions
L-proline-[procollagen] + Oxoglutaric acid + Oxygen → trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2)details
L-Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Succinic acid + Carbon dioxidedetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
General function:
Involved in iron ion binding
Specific function:
Catalyzes the formation of L-carnitine from gamma-butyrobetaine.
Gene Name:
BBOX1
Uniprot ID:
O75936
Molecular weight:
44714.6
Reactions
4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen → L-Carnitine + Succinic acid + CO(2)details
4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen → L-Carnitine + Succinic acid + Carbon dioxidedetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
  3. Yoshisue K, Yamamoto Y, Yoshida K, Saeki M, Minami Y, Esumi Y, Kawaguchi Y: Pharmacokinetics and biological fate of 3-(2,2, 2-trimethylhydrazinium)propionate dihydrate (MET-88), a novel cardioprotective agent, in rats. Drug Metab Dispos. 2000 Jun;28(6):687-94. [PubMed:10820142 ]
General function:
Involved in oxidoreductase activity
Specific function:
Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links.
Gene Name:
PLOD1
Uniprot ID:
Q02809
Molecular weight:
83549.55
Reactions
L-lysine-[procollagen] + Oxoglutaric acid + Oxygen → (2S,5R)-5-hydroxy-L-lysine-[procollagen] + Succinic acid + CO(2)details
Protein lysine + Oxoglutaric acid + Oxygen → Procollagen 5-hydroxy-L-lysine + Succinic acid + Carbon dioxide + Waterdetails
References
  1. Cudic M, Patel DA, Lauer-Fields JL, Brew K, Fields GB: Development of a convenient peptide-based assay for lysyl hydroxylase. Biopolymers. 2008;90(3):330-8. [PubMed:17610258 ]

Only showing the first 10 proteins. There are 44 proteins in total.